Many basal ganglia diseases due to different etiological factors cause parkinsonism. The main feature of Parkinsonism is the impoverishment of movement. This impoverishment may be in the form of a decrease in the amount of movement (hypokinesia), slowing down of movements (bradykinesia), pause or absence of movement (akinesia). Some authors also use hypokinesia to describe a decrease in the amplitude of movement. The four major characteristic features of the diagnosis of Parkinsonism are:Tremor,Rigidity,Akinesis andPostural instability ( >TRAP). Therefore, parkinsonism is generally the name given to the clinical syndrome in which these four major characteristics are found in different combinations.
Parkinson's disease (PD) is the most common variant of parkinsonism syndrome and is a movement disorder group. It is the most common disease after essential tremor. Clinicopathological studies have shown that the clinical syndrome that best reflects the typical pathological changes of PH is achieved by asymmetric disease with resting tremor and rigidity or bradykinesia, marked improvement with levodopa, and exclusion of other diagnoses known to cause parkinsonism. In recent years, many specific gene mutations causing PH clinic [a-synuclein (SNCA gene), Parkin (PARK2 gene), leucine-rich repeat [like the kinase 2 (LRRK2) gene] were detected. For this reason, it is accepted that the clinical picture called PH consists of many different diseases with common features. However, less than 10% of patients with PD have a genetic mutation. In addition to genetic causes, many other diseases, both pure parkinsonism and accompanied by other neurological deficits, also cause parkinsonism.
Many different disease presentations can mimic PD and cause difficulties in diagnosis. . The disease group that is most frequently problematic in diagnosis consists of diseases examined under the names “Parkinson-plus Syndromes” or “Atypical Parkinsonian Diseases”. In this group, “Multiple System Atrophy (MSA)”,
CLINICAL PICTURES THAT CAUSE PROBLEMS IN THE DIFFERENTIAL DIAGNOSIS OF PARKINSON'S DISEASE:
1.1 .Progressive Supranuclear Palsy (PSP)
PSP, also known as Steele-Richardson-Olszewski syndrome, is a sporadic and progressive neurodegenerative disease. PSP is the second most common atypical form of parkinsonism. The main findings that characterize PSP are vertical supranuclear ophthalmoplegia, early and significant postural instability, parkinsonism, axial rigidity, pseudobulbar palsy and mild dementia. PSP typically begins in the sixth or seventh decades of life, usually with gait and balance disorders and associated falls. Cognitive impairment is common and mostly in the frontal-subcortical form. Therefore, attention, executive dysfunction, and cued memory deficits are the dominant findings. Symmetrical akinesia and rigidity more pronounced in the proximal than the distal areas, limitation of eye movement, abnormal neck posture such as retrocollis, no or very little response to levodopa, and the presence of dysphagia, dysarthria and cognitive impairment findings in the early period are the criteria that support the disease. PSP shows a rapidly progressive course.
1.2.Multiple System Atrophy (MSA)
Multiple system atrophy (MSA)It is a clinical condition in which one or more of the autonomic, cerebellar and corticospinal findings are observed together with parkinsonism. This sporadic neurodegenerative disorder, which usually begins in the 6th decade, may be difficult to distinguish from PD, especially in the early stages. Today, MSA symptoms are divided into two main categories according to the dominant clinical symptom. In the majority of cases (80%) parkinsonism findings are dominant and this group, which was formerly classified as striatonigral degeneration, is now called MSA-P. In patients with MSA-P, progressive akinesia and rigidity generally predominate in the parkinsonism picture. It is quite difficult to distinguish it from Parkinson's disease in the early stages. It is difficult. However, classical rest tremor is either minimal or absent. While initial partial improvements due to levodopa can be seen in 30% of these patients, long-term unresponsiveness reaches up to 90%. The main pathology is in the striatonigral system. In 20% of cases with MSA, cerebellar findings are dominant, and this group, formerly known as olivopontocerebellar atrophy, is also classified as MSA-C. In patients with MSA-C, the main degeneration is located in the pons and cerebellar region. Ataxia, intentional tremor and nystagmus may be seen as symptoms of cerebellar dysfunction. Both MSA-P and MSA-C have signs of autonomic dysfunction such as orthostatic hypotension, impotence, urinary incontinence or retention. For this reason, it is thought that it does not make much sense to separately classify the MSA form in which autonomic involvement is dominant, which was formerly classified as Shy-Drager syndrome. Severe dementia is rarely seen in MSA cases and can even be considered an exclusion criterion. The combination of upper motor neuron findings, respiratory problems and parkinsonism findings is highly suggestive of MSA. Clinical diagnosis of MSAs can be made with history and physical examination findings. Cerebellar findings together with parkinsonism in which no obvious response to levodopa can be obtained, the detection of significant autonomic dysfunction or bulbar involvement findings in the early period, and the absence of accompanying dementia largely support the diagnosis of MSA.
1.3.Corticobasal. Degeneration (CBD)
Although the onset of CBD is clinically heterogeneous, the typical clinical picture is akinetic rigid syndrome that is unresponsive to levodopa and has an asymmetric onset, with findings such as apraxia, dystonia, myoclonus, cortical sensory impairment and foreign limb syndrome. Contains. The average age of onset is between 60-64 years. The most common form is predominantly a motor disease, but patients can also present with primary progressive aphasia and develop global dementia. Aphasia is observed in more than half of the patients. The presence of akinetic parkinsonism along with significant asymmetric rigidity and ipsilateral apraxia and dystonia in the early stages should suggest CBD. Although alien limb syndrome is a unique finding, it is relatively rare. e occurs in more advanced stages. Dystonic posture in the hand and arm may occur in half of the patients. Tremor is not common; if there is tremor, it is in the form of action tremor that improves with rest. Frontal lobe type behavioral changes such as apathy, social shyness, abnormal behavior, increased sexual desire and irritability are present at a rate of 30-50%. Eye movement limitations may be detected, affecting both vertical and horizontal eye movements equally. Brain imaging of KBD patients often shows focal cortical atrophy with widening of asymmetric sylvian and interhemispheric fissures and dilatation of frontal, temporal and parietal sulci, consistent with clinical involvement. The prognosis is progressive.
1.4.Dementia with Lewy Bodies (LCD)
Dementia with Lewy bodies (LCD) is a disease of Alzheimer's disease. It is the second most common degenerative dementia. In particular, it is a progressive dementia characterized by fluctuating cognitive impairment, predominant impairment of attention and visuospatial abilities, visual hallucinations and parkinsonism. Dementia called Parkinson's disease dementia (PHD) occurs in the majority of Parkinson's patients, especially in the late stages of the disease. However, the condition in which dementia is the first manifestation or occurs within the first year following parkinsonism is considered LCD. Similar features can be seen in PHD and LCD. However, the fact that axial involvement is more prominent in the distribution of extrapyramidal symptoms on LCD and less tremor is seen may be distinguishing. More importantly, there are differences in the temporal development of disease symptoms in these two diseases. The three most important clinical findings in Lewy body dementia are fluctuating cognitive performance, visual hallucinations, and parkinsonism findings. These patients have lower tolerance to dopaminergic treatment. Even low doses of dopaminergic treatments can cause psychotic symptoms.
1.5.Parkinsonism and Frontotemporal Degeneration associated with Chromosome 17 (FTDP-17)< br />
Frontotemporal degeneration is a group of diseases characterized by behavioral changes and neuropsychological evidence of frontal lobe dysfunction. �r. FTDP-17 clinic includes dementia and parkinsonism, which manifest with significant behavioral changes such as disinhibition, apathy, impaired judgment, compulsive behaviors, aggression, and hyperorality.
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